Scaphocephalus

Classification according to ICD-10
Q75.0	Craniosynostosis
ICD-10 online (WHO version 2019)

A scaphocephalus , also Scaphocephalus or Kahnskull ( ancient Greek σκαφίς skaphis 'Kahn', κεφαλή kephalē 'head') is the most common form of craniosynostosis , a premature ossification of cranial sutures . In scaphocephalus there is a premature ossification of the arrow suture (sagittal suture , sutura sagittalis ), which results in an elongated, narrow skull shape with a high forehead. For the surgical correction of the malformation, the craniotomy is used in the child's first months of life. As a rule, surgery is performed for cosmetic reasons; there is seldom an indication due to increased intracranial pressure .

The malformation usually occurs in isolation ( isolated scaphocephaly ), only rarely does it occur in connection with other diseases such as I-cell disease (mucolipidosis II).

The isolated malformation occurs mostly sporadically with a frequency of 1: 5000 live births; in 6% of the cases it is inherited as an autosomal dominant trait . Its cause is unknown. The intelligence of the affected children is usually not impaired.

literature

B. Kotrikova, J. Mühling: Surgery of craniofacial malformations. In: Clinical monthly sheets for ophthalmology. Volume 221, Number 11, November 2004, pp. 970-977, ISSN 0023-2165 . doi: 10.1055 / s-2004-813654 . PMID 15562363 . (Review article).

Individual evidence

↑ Joachim E. Zöller, Alexander C. Kübler, Wilma D. Lorber and Joachim Mühlin: Craniofacial surgery. Thieme, Stuttgart 2003, ISBN 3-13-131391-9 , p. 12f.
↑ Joachim E. Zöller, Alexander C. Kübler, Wilma D. Lorber, Joachim Mühling: Craniofacial surgery. Thieme, Stuttgart 2003, ISBN 3-13-131391-9 , p. 28, p. 89f.
↑ Dag Moskopp, Hansdetlef Wassmann: Neurosurgery. Schattauer, 2004, ISBN 3-7945-1991-4 , pp. 730ff.
^ S. Kornfeld, WS Sly: I-cell disease and pseudo-Hurler polydystrophy: Disorders of lysosomal enzyme phosphorylation and localization. In: The Metabolic and Molecular Bases of Inherited Disease. Volume 2. McGraw-Hill, New York 1995, pp. 2495-2508.
↑ Isolated scaphokephaly from Orphanet , accessed November 16, 2012.

[1] Joachim E. Zöller, Alexander C. Kübler, Wilma D. Lorber and Joachim Mühlin: Craniofacial surgery. Thieme, Stuttgart 2003, ISBN 3-13-131391-9 , p. 12f.

[2] Joachim E. Zöller, Alexander C. Kübler, Wilma D. Lorber, Joachim Mühling: Craniofacial surgery. Thieme, Stuttgart 2003, ISBN 3-13-131391-9 , p. 28, p. 89f.

[3] Dag Moskopp, Hansdetlef Wassmann: Neurosurgery. Schattauer, 2004, ISBN 3-7945-1991-4 , pp. 730ff.

[4] S. Kornfeld, WS Sly: I-cell disease and pseudo-Hurler polydystrophy: Disorders of lysosomal enzyme phosphorylation and localization. In: The Metabolic and Molecular Bases of Inherited Disease. Volume 2. McGraw-Hill, New York 1995, pp. 2495-2508.

[5] Isolated scaphokephaly from Orphanet , accessed November 16, 2012.